Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: Comparison of CT features
Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: Comparison of CT features
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DOI：
10.1148/radiol.2421051767
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发表时间：
2007-01-01
期刊：
影响因子：
19.7
通讯作者：
Moss, Joel
中科院分区：
文献类型：
--
作者：
Avila, Nilo A.;Dwyer, Andrew J.;Moss, Joel
目的：回顾性比较淋巴管-平滑肌瘤病(LAM)和结节性硬化症(TSC)及LAM患者的CT表现。材料和方法：HIPAA-遵从性研究需获得机构评审委员会的批准和知情同意。在256名LAM患者(平均年龄44岁)和67名TSC/LAM患者(平均年龄40岁)中，由一名放射科医生检查胸部、腹部和骨盆的CT扫描。通过高空间分辨率CT扫描估计囊性病变的肺部受累程度。评估的其他发现包括非钙化性肺结节、胸腔积液、胸导管扩张、肝和肾血管肌瘤(AML)、淋巴管肌瘤(LALM)、腹水、肾切除术和肾栓塞术。结果：LAM患者肺部受累范围更广(RIDIT评分，0.36)，LALM(29%比9%，P<；.001)，胸管扩张(4%比0，P=.3)，胸腔积液(12%比6%，P=.2)和腹水(10%比6%，P=.3)。非钙化肺结节(12%vs 1%，P&t；.01)、肝脏(33%vs 2%，P<；.001)、肾脏(93%vs 32%，P<；.001)、肾切除术(25%vs 7%，P<；.001)、肾动脉栓塞术(9%vs 2%，P<；.05)发生率较高。肝、肾AML和非钙化肺结节在TSC/LAM中更常见，而淋巴管受累-胸管扩张、乳糜性胸腔积液、腹水和LALM-在LAM中更常见。(C)RSNA，2006。
Purpose: To retrospectively compare the frequencies of computed tomographic (CT) findings in patients with lymphangio-leiomyomatosis (LAM) and patients with tuberous sclerosis complex (TSC) and LAM.Materials and Methods: Institutional review board approval and informed consent were obtained for the HIPAA-compliant study. In 256 patients with LAM (mean age, 44 years) and 67 patients with TSC/LAM (mean age, 40 years), CT scans of the chest, abdomen, and pelvis were reviewed by a single radiologist. The fraction or lung involvement with cysts was estimated from high-spatial-resolution CT scans. Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angio-myolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. Confidence intervals and hypothesis tests of differences in frequencies, comparison of age quartiles, RIDIT analysis, analysis of variance, and correlation coefficients were used in the statistical analysis.Results: Patients with LAM had more extensive lung involvement (RIDIT score, 0.36) and higher frequency of LALM (29% vs 9%, P < .001), thoracic duct dilatation (4% vs 0, P = .3), pleural effusion (12% vs 6%, P = .2), or ascites (10% vs 6%, P = .3). Patients with TSC/LAM had higher frequency of noncalcified pulmonary nodules (12% vs 1%, P < .01), hepatic (33% vs 2%, P < .001) and renal (93% vs 32%, P < .001) AMLs, nephrectomy (25% vs 7%, P < .001), or renal artery embolization (9% vs 2%,,P < .05).Conclusion: The extent of lung disease is greater in LAM than TSC/LAM. Hepatic and renal AMLs and noncalcified lung nodules are more common in TSC/LAM, while lymphatic involvement-thoracic duct dilatation, chylous pleural effusion, ascites, and LALM-is more common in LAM.(c) RSNA, 2006.